Multiple anomalous left pulmonary venous connections detected with transthoracic echocardiography
Chang- Chyi Lin
Heart Center, Cheng Hsin General Hospital
No. 45, Cheng Hsin St, Pai-Tou, Taipei
Shin Kong Wu Ho-Su Memorial Hospital
Partial anomalous pulmonary venous connection is a rare congenital anomaly in which one or more pulmonary veins are connected to the venous circulation. The condition is frequently misdiagnosed, and usually identified by transesophageal echocardiography or invasive cardiac catheterization. We present the case of a 26-year-old female with new onset dyspnea on exertion who was diagnosed with the left superior and inferior pulmonary veins draining into the innominate vein via a vertical vein by two and three-dimensional transthoracic echocardiography and multidetector computed tomographic angiography.
Partial anomalous pulmonary venous connection (PAPVC)
is a rare congenital anomaly in which pulmonary veins
carry blood from the lungs to the right side of the heart.
The condition has a prevalence of 0.4-0.7%, it is
frequently diagnosed as an incidental finding, and right
side PAPVC is more common than left [1,2] A single
PAPVC is found in 53% of cases, and two in 42%; more
numerous anomalous connections are very rare 
Herein we report an adult patient in which both the left
superior and inferior pulmonary veins (LSPV and LIPV)
drained into the innominate vein that was diagnosed by
non-invasive modalities: transthoracic echocardiography
(TTE) and multidetector computed tomographic (CT)
A 26-year-old female with no history of cardiovascular
disease was referred to our hospital for mild dyspnea
during exertion over a period of 3 months. She had been
treated at another institution with propranolol and
alprazolam for a presumptive diagnosis of mitral valve
prolapse without relief. On admission her blood pressure
was 110/64 mm Hg, heart rate was 86 beats/min, and
respiratory rate was 18 breaths/min. Physical
examination was unremarkable. A 12-lead electrocardiogram
showed normal sinus rhythm with incomplete right
bundle branch block. Chest radiography was normal.
TTE showed normal left and right ventricular function,
but the right ventricle was mildly dilated. Right
ventricular systolic pressure was 35 mm Hg with moderate
tricuspid regurgitation. There was no evidence of an
interatrial septal defect, although the calculated Qp/Qs
was 1.85. Suprasternal short-axis view revealed direct
drainage of both the LSPV and LIPV into the
innominate vein (IV) via a vertical vein (VV) (Figure 1A). Color
Doppler imaging in the same plane also showed red
flow in IV and VV (directed toward the transducer) and
blue flow in superior vena cava (directed away from the
transducer) (Figure 1B) Pulse Doppler showed a venous
flow pattern upward in the vertical vein (Figure 1C).
Multidetector CT angiography was performed to to
confirm the echocardiographic findings of anomalous
circulation (Figure 1D). She underwent surgical
correction during which the distal end of the vertical vein was
anastomosed to the left atrial appendage (Figure 2).
Postoperative echocardiography showed the Qp/Qs below
1.0, and her symptoms had resolved after 6-months
Figure 1 Four images for detecting partial anomalous pulmonary venous connection (PAPVC). (A) Three dimensional transthoracic
echocardiography showed PAPVC. (B) The suprasternal short-axis view with color Doppler revealed left pulmonary veins connecting with the
innominate vein via a vertical vein. (C) Upward blood flow in the vertical vein was confirmed by pulsed Doppler imaging. (D) Multidetector
computed tomography angiography was performed to confirm the structure of the anomalous venous system. IV: innominate vein; SVC: superior
vena cava; VV: vertical vein.
PAPVC is an important diagnosis, but is often overlooked
in clinical practice. Early presentations resemble those
of patients with an atrial septal defect (ASD), exercise
intolerance, or atrial arrhythmia, and later in the course
of the disease, right heart failure and pulmonary
hypertension. Symptoms relate to the magnitude of left-to-right
shunting . The most common associated anomaly is an
ASD [2,4]. The reported incidence of sinus venosus ASD
in patients with PAPVC ranges from 49% to 85%, and that
of secundum ASD ranges from 10% to 33% [2,4-6].
Though rare, cases of PAPVC associated with valvular
heart disease have been reported . It is important to
understand whether the anomalous pulmonary
connection coexisted with or without an interatrial shunt
before planning the surgical correction. The reported
sites of PAPVC include the right superior vena cava, left
subclavian vein, left innominate vein, azygous vein, portal
vein, and coronary sinus .
Invasive cardiac catheterization and transesophageal
echocardiography are often used to diagnosis
pulmonary vein return anomalies; however, the procedure is
performed under sedation and is associated with inherent
risks. In this case, TTE efficiently detected the anomaly in
suprasternal short-axis view. TTE can identify right atrial
and ventricular enlargement, increased pulmonary
pressure, and interventricular septal flattening as a result of
increased right ventricular pressure . The suprasternal
and subcostal views are very important in diagnosing
PAPVC. For example, subcostal view can identify
pulmonary venous connections and septal abnormalities
and the suprasternal short-axis view offer excellent
Figure 2 Surgical correction of the partial anomalous pulmonary venous connection (PAPVC). The diagram of PAPVC (left). The distal end
of the vertical vein was anastomosed to the left atrial appendage (right). SVC: superior vena cava; LIV: left innominate vein; VV: vertical vein; LSPV:
Left superior pulmonary vein; LIPV: Left inferior pulmonary vein; RPV: Right pulmonary vein;PA: pulmonary artery; RA: Right atrium; RV: Right
ventricle; LV: left ventricle.
imaging of all four pulmonary veins and can identify
abnormal structures such as a vertical vein with upward
blood flow . Nevertheless, it is more challenging to
perform these views of echocardiography in adults than
in children because of grown body size and body
composition. Several conditions, for example, obesity, chronic
obstructive pulmonary disease, and chest wall deformities
can also reduce the accuracy of TTE. Patients with
unexplained right heart dilatation should prompt the
sonographer to search extensively for evidence of a left-to-right
shunt, most likely across an ASD, and/or for the presence
of PAPVC. Additionally, in this patient while Qp:Qs > 1
there was no evidence of flow across the interatrial
septum; thus, the findings were suspicious for a PAPVC
and then an comprehensive searching for pulmonary
venous connections should be performed.
As a complement to TTE, color Doppler and
threedimensional echocardiography are very useful for
identifying the presence of these abnormalities . In addition,
CT can provide optimal visualization of the complete
thoracic vessels by reconstruction, as well as cardiac
magnetic resonance imaging .
Surgical correction is necessary in cases where Qp/Qs
is > 1.5:1 and the right ventricle is enlarged . Reports
of repair in adults have indicated good outcomes .
The degree of left-to-right shunting determines the type
of management . Patients with anomalous drainage
of more than 50% of the pulmonary blood flow should
have surgical repair to prevent following complications.
For patients with more than one anomalous pulmonary
venous connection but less than 50% anomalous
drainage of pulmonary blood flow, the degree of left-to-right
shunting may increase during adulthood because of
changes in left ventricular compliance. These patients
should remain under periodic review and surgery is
indicated while symptoms such as dyspnea on exertion
develop. Asymptomatic cases such as isolated PAPVC
with low level of Qp/Qs can be managed conservatively
with annual follow-up .
Partial anomalous pulmonary venous connection is a rare
and frequently misdiagnosed congenital anomaly in which
pulmonary veins are connected to the venous circulation.
We present the case of a 26-year-old female with new
onset dyspnea diagnosed with the left superior and inferior
pulmonary veins draining into the innominate vein by
two and three-dimensional transthoracic echocardiography
and multidetector computed tomographic angiography.
Written informed consent was obtained from the patient
for publication of this Case report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
PAPVC: Partial anomalous pulmonary venous connection; LIPV: Left inferior
pulmonary vein; LSPV: Left superior pulmonary vein; TTE: Transthoracic
echocardiography; CT: Computed tomography; IV: Innominate vein;
VV: Vertical vein; ASD: Atrial septal defect.
TLW drafted the manuscript and performed literature search. HFH and CCL
supervised and reviewed the manuscript. MCH performed echocardiography,
analyzed and interpreted the imaging results and supervised the manuscript.
JW performed the surgery and coordinated the study. All authors read and
approved the final manuscript.